MCID: VRS001
MIFTS: 32

Virus Associated Hemophagocytic Syndrome

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Virus Associated Hemophagocytic Syndrome

MalaCards integrated aliases for Virus Associated Hemophagocytic Syndrome:

Name: Virus Associated Hemophagocytic Syndrome 52

Classifications:



Summaries for Virus Associated Hemophagocytic Syndrome

NIH Rare Diseases : 52 Virus associated hemophagocytic syndrome is a very serious complication of a viral infection. Signs and symptoms of virus associated hemophagocytic syndrome, include high fever, liver problems , enlarged liver and spleen, coagulation factor abnormalities, decreased red or white blood cells and platelets (pancytopenia), and a build-up of histiocytes, a type of immune cell , in various tissues in the body resulting in the destruction of blood-producing cells (histiocytic proliferation with prominent hemophagocytosis). Diagnosis is based upon the signs and symptoms of the patient. The cause of the condition is not known. Treatment is challenging and approach will vary depending on the age and medical history of the patient. Complications of this syndrome can become life threatening. Related conditions (conditions with overlapping signs and symptoms), include histiocytic medullary reticulosis (HMR), familial hemophagocytic lymphohistiocytosis (FHL), and X-linked lymphoproliferative syndrome .

MalaCards based summary : Virus Associated Hemophagocytic Syndrome is related to chickenpox and exanthem. An important gene associated with Virus Associated Hemophagocytic Syndrome is CD68 (CD68 Molecule), and among its related pathways/superpathways are IL-2 Pathway and Common Cytokine Receptor Gamma-Chain Family Signaling Pathways. The drugs Lenograstim and Methotrexate have been mentioned in the context of this disorder. Affiliated tissues include liver, spleen and bone marrow, and related phenotype is hematopoietic system.

Related Diseases for Virus Associated Hemophagocytic Syndrome

Diseases related to Virus Associated Hemophagocytic Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 212)
# Related Disease Score Top Affiliating Genes
1 chickenpox 30.3 IL4 IL2
2 exanthem 30.2 PIK3C2A IL2
3 encephalopathy, progressive, early-onset, with episodic rhabdomyolysis 29.8 PIK3C2A MB
4 rubella 29.6 IL4 IL2
5 acute kidney failure 29.6 PIK3C2A MB
6 disseminated intravascular coagulation 29.5 PIK3C2A MB
7 immune deficiency disease 29.5 IL4 IL2RB IL2
8 pneumonia 28.4 PIK3C2A MB IL4 IL2
9 myocarditis 27.9 PIK3C2A MB IL4 IL2 CD68
10 hemophagocytic syndrome associated with an infection 11.9
11 pancytopenia 10.5
12 histiocytosis 10.4
13 fibrosarcoma of bone 10.4
14 pfeiffer syndrome 10.3
15 thrombocytopenia 10.3
16 splenomegaly 10.2
17 granulomatous myositis 10.2 PIK3C2A CD68
18 neutropenia 10.2
19 berylliosis 10.1 IL2RB IL2
20 boutonneuse fever 10.1 IL2RB IL2
21 central nervous system lymphoma 10.1 IL2 CD68
22 b-cell expansion with nfkb and t-cell anergy 10.1 IL2RB IL2
23 erysipelas 10.1 PIK3C2A IL2
24 necrotizing fasciitis 10.1 PIK3C2A IL2
25 hemophagocytic lymphohistiocytosis 10.1
26 respiratory failure 10.1
27 herpes simplex 10.1
28 t-cell large granular lymphocyte leukemia 10.1 IL2RB IL2
29 alzheimer disease 17 10.1 IL4 CD68
30 1-chloro-2,4-dinitrobenzene allergic contact dermatitis 10.1 IL4 IL2
31 contact dermatitis 10.1 IL4 IL2
32 jak3-deficient severe combined immunodeficiency 10.1 IL4 IL2
33 atopic keratoconjunctivitis 10.1 IL4 IL2
34 keratoconjunctivitis 10.1 IL4 IL2
35 conjunctivitis 10.1 IL4 IL2
36 angiostrongyliasis 10.0 IL4 IL2
37 autoimmune vasculitis 10.0 IL4 IL2
38 mucocutaneous leishmaniasis 10.0 IL4 IL2
39 cutaneous leishmaniasis 10.0 IL4 IL2
40 eosinophilic meningitis 10.0 IL4 IL2
41 autoimmune disease of eyes, ear, nose and throat 10.0 IL4 IL2
42 primary systemic mycosis 10.0 IL4 IL2
43 periapical granuloma 10.0 IL4 IL2
44 microscopic colitis 10.0 IL4 IL2
45 lichen disease 10.0 IL4 IL2
46 autoimmune gastritis 10.0 IL4 IL2
47 immunodysregulation, polyendocrinopathy, and enteropathy, x-linked 10.0 IL4 IL2
48 erythema multiforme 10.0 IL4 IL2
49 autoimmune disease of blood 10.0 IL4 IL2
50 vulvovaginal candidiasis 10.0 IL4 IL2

Graphical network of the top 20 diseases related to Virus Associated Hemophagocytic Syndrome:



Diseases related to Virus Associated Hemophagocytic Syndrome

Symptoms & Phenotypes for Virus Associated Hemophagocytic Syndrome

MGI Mouse Phenotypes related to Virus Associated Hemophagocytic Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.1 CD68 IL2 IL2RB IL4 MB PIK3C2A

Drugs & Therapeutics for Virus Associated Hemophagocytic Syndrome

Drugs for Virus Associated Hemophagocytic Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 43)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Lenograstim Approved, Investigational 135968-09-1
2
Methotrexate Approved 59-05-2, 1959-05-2 126941
3
Etoposide Approved 33419-42-0 36462
4
leucovorin Approved 58-05-9 6006 143
5
Clotrimazole Approved, Vet_approved 23593-75-1 2812
6
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
7
Busulfan Approved, Investigational 55-98-1 2478
8
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
9
Methylprednisolone Approved, Vet_approved 83-43-2 6741
10
Prednisone Approved, Vet_approved 53-03-2 5865
11
Prednisolone phosphate Approved, Vet_approved 302-25-0
12
Methylprednisolone hemisuccinate Approved 2921-57-5
13 Prednisolone acetate Approved, Vet_approved 52-21-1
14
Prednisolone Approved, Vet_approved 50-24-8 5755
15
Folic acid Approved, Nutraceutical, Vet_approved 59-30-3 6037
16
Prednisolone hemisuccinate Experimental 2920-86-7
17 Immunologic Factors
18 Etoposide phosphate
19 Immunosuppressive Agents
20 Vitamin B Complex
21 Alkylating Agents
22 Antifungal Agents
23 Folic Acid Antagonists
24 Folate
25 Anti-Infective Agents
26 Dermatologic Agents
27 Cyclosporins
28 Calcineurin Inhibitors
29 Antirheumatic Agents
30 Antimetabolites
31 Antilymphocyte Serum
32 Vitamin B9
33 Gastrointestinal Agents
34 Methylprednisolone Acetate
35 Hormones
36 Complement System Proteins
37 Antineoplastic Agents, Hormonal
38 Antiemetics
39 Hormone Antagonists
40 Protective Agents
41 glucocorticoids
42 Anti-Inflammatory Agents
43 Neuroprotective Agents

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Pilot Study of Unrelated Donor Hematopoietic Stem Cell Transplantation in Patients With Life Threatening Hemophagocytic Disorders Unknown status NCT00006056 anti-thymocyte globulin;busulfan;cyclophosphamide;cyclosporine;etoposide;filgrastim;methotrexate
2 Allogeneic Bone Marrow Transplantation in Patients With Primary Immunodeficiencies Terminated NCT00006054 anti-thymocyte globulin;busulfan;cyclophosphamide;cyclosporine;etoposide;methotrexate;methylprednisolone;prednisone

Search NIH Clinical Center for Virus Associated Hemophagocytic Syndrome

Genetic Tests for Virus Associated Hemophagocytic Syndrome

Anatomical Context for Virus Associated Hemophagocytic Syndrome

MalaCards organs/tissues related to Virus Associated Hemophagocytic Syndrome:

40
Liver, Spleen, Bone Marrow, Bone, T Cells, Heart, Lung

Publications for Virus Associated Hemophagocytic Syndrome

Articles related to Virus Associated Hemophagocytic Syndrome:

(show top 50) (show all 231)
# Title Authors PMID Year
1
Early Use of Blood Purification in Severe Epstein-Barr Virus-Associated Hemophagocytic Syndrome. 61
32430444 2020
2
[Clinical Characteristics and Prognostic Analysis of 59 Adult Patients with Epstein-Barr Virus-Associated Hemophagocytic Syndrome]. 61
32319412 2020
3
Echovirus Type 7 Virus-Associated Hemophagocytic Syndrome in a Neonate Successfully Treated With Intravenous Immunoglobulin Therapy: A Case Report. 61
31799224 2019
4
Epstein-Barr virus-associated hemophagocytic syndrome in a patient with ulcerative colitis during treatment with azathioprine: A case report and review of literature. 61
30510942 2018
5
Infusion of leukocytes from HLA haplo-identical familial donors as an adjuvant in the HLH-2004 protocol to treat the virus-associated adult hemophagocytic lymphohistiocytosis: a retrospective study of 26 patients. 61
29130133 2018
6
Inherited Immunodeficiencies With High Predisposition to Epstein-Barr Virus-Driven Lymphoproliferative Diseases. 61
29942301 2018
7
Severe dengue due to secondary hemophagocytic lymphohistiocytosis: A case study. 61
28409119 2017
8
Epstein-Barr Virus Associated Hemophagocytic Syndrome after Scrub Typhus Infection. 61
27883370 2016
9
Fulminant Epstein-Barr virus-associated hemophagocytic syndrome in a renal transplant patient and review of the literature. 61
27496079 2016
10
Disruption of vascular endothelial homeostasis in systemic juvenile idiopathic arthritis-associated macrophage activation syndrome: The dynamic roles of angiopoietin-1 and -2. 61
26908294 2016
11
Dengue fever complicated by hemophagocytosis. 61
27274854 2016
12
[Successful Treatment of Seasonal Influenza A (H3N2) infection-related Hemophagocytic Lymphocytosis in an Elderly Man]. 61
27032178 2016
13
Analysis of prognostic risk factors in children with Epstein-Barr virus-associated hemophagocytic syndrome. 61
25941132 2015
14
[The use of therapeutic play in the intensive care of a preschool child with virus-associated hemophagocytic syndrome]. 61
25854953 2015
15
[Allogeneic hematopoietic stem cell transplantation for Epstein-Barr virus associated hemophagocytic syndrome: a case report and literatures review]. 61
25916301 2015
16
Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis under Treatment with Tocilizumab. 61
25684767 2015
17
Virus-associated hemophagocytic syndrome in renal transplant recipients: report of 2 cases from a single center. 61
25838952 2015
18
[A case report of giardia lamblia and EB virus associated hemophagocytic syndrome]. 61
25523583 2014
19
Evidence for human herpesvirus-6B infection of regulatory T-cells in acute systemic lymphadenitis in an immunocompetent adult with the drug reaction with eosinophilia and systemic symptoms syndrome: a case report. 61
25249343 2014
20
Hemophagocytic syndrome in severe dengue Fever: a rare presentation. 61
25332549 2014
21
Systematic Epstein-Barr virus-positive T-cell lymphoproliferative disease presenting as a persistent fever and cough: a case report. 61
25163591 2014
22
BK virus-associated hemophagocytic syndrome in a renal transplant recipient. 61
24821160 2014
23
[Case report: a case of Epstein-Barr virus associated hemophagocytic syndrome with reversible leukoencephalopathy in the splenium of the corpus callosum]. 61
23947245 2013
24
[Clinical analysis of Epstein-Barr virus-associated hemophagocytic syndrome in children]. 61
23628054 2013
25
Hemophagocyctic lymphohistiocytosis developed in a Japanese boy with Chédiak-Higashi syndrome. 61
23994801 2013
26
Cytokine nephropathy in a patient with fatal Epstein-Barr virus-associated hemophagocytic syndrome. 61
23991898 2013
27
A case of novel swine influenza A (H1N1) pneumonia complicated with virus-associated hemophagocytic syndrome. 61
22286408 2012
28
The risk factors of fungal infection in living-donor liver transplantations. 61
21792556 2012
29
Ocular findings in a patient with virus-associated hemophagocytic syndrome. 61
22486263 2012
30
[Neonatal herpes simplex type II virus infection complicated with meningitis and virus-associated hemophagocytic syndrome]. 61
22374449 2012
31
Repeated Epstein-Barr virus-associated hemophagocytic syndrome in a lupus nephritis patient. 61
22185972 2012
32
[Research advance on Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis]. 61
22169321 2011
33
Hemophagocytic syndrome in classic dengue Fever. 61
22224008 2011
34
[Virus-associated hemophagocytic syndrome (VAHS) as an initial manifestation in HIV infection]. 61
21919309 2011
35
Hemophagocytic syndrome secondary to cytomegalovirus infection in an infant. 61
21552407 2011
36
Virus-associated hemophagocytic syndrome as a major contributor to death in patients with 2009 influenza A (H1N1) infection. 61
21366922 2011
37
Virus-associated hemophagocytic syndrome caused by pandemic swine-origin influenza A (H1N1) in a patient after unrelated bone marrow transplantation. 61
21628863 2011
38
Resolution of Henoch-Schönlein purpura nephritis after acquired IgA deficiency. 61
20526852 2010
39
Hemophagocytic syndrome in a patient with acute tubulointerstitial nephritis secondary to hepatitis A virus infection. 61
20890439 2010
40
Juvenile idiopathic arthritis presenting with prolonged fever. 61
21291842 2010
41
Novel swine-origin influenza A (H1N1) virus-associated hemophagocytic syndrome--a first case report. 61
20348529 2010
42
Dengue virus associated hemophagocytic syndrome in children: a case report. 61
20853575 2010
43
Kaposi sarcoma herpes virus-associated hemophagocytic syndrome complicated by multicentric castleman disease and kaposi sarcoma in a HIV-negative immunocompetent patient: an autopsy case. 61
19795003 2009
44
Virus-Associated Hemophagocytic Syndrome related to acute CMV and HBV sexual co-infection: a case report. 61
19640781 2009
45
Unique phenotypes of C1s deficiency and abnormality caused by two compound heterozygosities in a Japanese family. 61
19155518 2009
46
Epstein-Barr virus-induced infectious mononucleosis after two separate episodes of virus-associated hemophagocytic syndrome. 61
19571453 2009
47
[Viral form of hemophagocytic syndrome with erythrodermal clinical picture--case report]. 61
19097380 2008
48
Epstein-Barr virus-associated hemophagocytic syndrome mimicking severe sepsis. 61
19561991 2008
49
[Case of domestically infected hepatitis E with marked thrombocytopenia]. 61
18525191 2008
50
Hemophagocytic syndrome associated with hepatitis A: case report and literature review. 61
18488094 2008

Variations for Virus Associated Hemophagocytic Syndrome

Expression for Virus Associated Hemophagocytic Syndrome

Search GEO for disease gene expression data for Virus Associated Hemophagocytic Syndrome.

Pathways for Virus Associated Hemophagocytic Syndrome

Pathways related to Virus Associated Hemophagocytic Syndrome according to GeneCards Suite gene sharing:

(show all 19)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.84 PIK3C2A IL4 IL2RB IL2
2
Show member pathways
12.42 IL4 IL2RB IL2
3
Show member pathways
12.24 IL4 IL2RB IL2
4 12.2 IL4 IL2RB IL2
5
Show member pathways
12.11 IL4 IL2RB IL2
6
Show member pathways
12.01 PIK3C2A IL2RB IL2
7
Show member pathways
11.73 IL4 IL2RB IL2
8
Show member pathways
11.63 IL4 IL2RB IL2
9 11.5 IL4 IL2
10
Show member pathways
11.46 IL4 IL2
11 11.45 IL4 IL2RB IL2
12
Show member pathways
11.34 IL2RB IL2
13
Show member pathways
11.26 IL4 IL2RB IL2
14
Show member pathways
11.05 IL2RB IL2
15 10.99 IL4 IL2RB IL2
16 10.99 IL4 IL2RB IL2 CD68
17 10.94 IL4 IL2
18 10.85 IL4 IL2
19 10.65 IL4 IL2RB IL2

GO Terms for Virus Associated Hemophagocytic Syndrome

Biological processes related to Virus Associated Hemophagocytic Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine-mediated signaling pathway GO:0019221 9.5 IL4 IL2RB IL2
2 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.46 IL4 IL2
3 positive regulation of T cell proliferation GO:0042102 9.43 IL4 IL2
4 positive regulation of B cell proliferation GO:0030890 9.4 IL4 IL2
5 extrinsic apoptotic signaling pathway in absence of ligand GO:0097192 9.37 IL4 IL2
6 positive regulation of T cell differentiation GO:0045582 9.26 IL4 IL2
7 interleukin-2-mediated signaling pathway GO:0038110 9.16 IL2RB IL2
8 positive regulation of isotype switching to IgG isotypes GO:0048304 8.96 IL4 IL2
9 negative regulation of T-helper 17 cell differentiation GO:2000320 8.62 IL4 IL2

Sources for Virus Associated Hemophagocytic Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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